Argininosuccinate synthetase 1, Polyclonal Antibody

Goat Anti-Argininosuccinate synthetase 1, Biotinylated Antibody; Argininosuccinate synthetase 1; ASS; ASS1; CTLN1; citrulline--aspartate ligase; Argininosuccinate synthetase 1 antibody; ASS antibody; ASS1 antibody; CTLN1 antibody; citrulline--aspartate ligase antibody; Argininosuccinate synthetase 1; Argininosuccinate synthetase 1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-ASS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Peptide ELISA (EIA), Western Blot (WB), Immunofluorescence (IF), Immunoprecipitation (IP), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 64000.
Immunohistochemistry: In paraffin embedded Human Kidney shows textured cytoplasm staining in PCT. Recommended concentration, 2-4ug/ml.
Western Blot: Approx 45kDa band observed in Human Kidney lysates (calculated MW of 46.5kDa according to NP_000041.2 and NP_446464.1). Recommended concentration: 0.01-0.03ug/ml.
Immunofluorescence: A customer has reported this antibody works well in IF.
Immunoprecipitation: This antibody has been successfully used in IP from BAEC cells as described in the following paper: Corbin et al, Biochem Biophys Res Commun.2008 Dec 26;377(4): 1042-6, PMID: 18948083.

(0. 03ug/ml) staining of Human Kidney lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

(2.5ug/ml) staining of paraffin embedded Human Kidney. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.

46,530 Da

argininosuccinate synthase 1

argininosuccinate synthase

Argininosuccinate synthase

ASS  [Similar Products]

ASSY_HUMAN

The protein encoded by this gene catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase. Mutations in the chromosome 9 copy of this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]

ASS1: Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1). Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or *****hood. Belongs to the argininosuccinate synthase family. Type 1 subfamily.

Protein type: Amino Acid Metabolism - arginine and proline; Endoplasmic reticulum; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 6.3.4.5; Mitochondrial; Ligase

Chromosomal Location of Human Ortholog: 9q34.1

Cellular Component: cytoplasm; cytosol; endoplasmic reticulum; lysosome; mitochondrial outer membrane; nucleus; perikaryon

Molecular Function: amino acid binding; argininosuccinate synthase activity; ATP binding; identical protein binding; protein binding; toxin binding

Biological Process: acute-phase response; aging; arginine biosynthetic process; argininosuccinate metabolic process; aspartate metabolic process; citrulline metabolic process; kidney development; liver development; midgut development; positive regulation of nitric oxide biosynthetic process; response to drug; response to estradiol stimulus; response to mycotoxin; response to nutrient; response to zinc ion; urea cycle

Disease: Citrullinemia, Classic

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